New insights into the role of HNF-1 β in kidney (patho)physiology

AbstractHepatocyte nuclear factor-1 β (HNF-1β) is an essential transcription factor that regulates the development and function of epithelia in the kidney, liver, pancreas, and genitourinary tract. Humans who carryHNF1B mutations develop heterogeneous renal abnormalities, including multicystic dysplastic kidneys, glomerulocystic kidney disease, renal agenesis, renal hypoplasia, and renal interstitial fibrosis. In the embryonic kidney, HNF-1 β is required for ureteric bud branching, initiation of nephrogenesis, and nephron segmentation. Ablation of mouseHnf1b in nephron progenitors causes defective tubulogenesis, whereas later inactivation in elongating tubules leads to cyst formation due to downregulation of cystic disease genes, includingUmod,Pkhd1, andPkd2. In the adult kidney, HNF-1 β controls the expression of genes required for intrarenal metabolism and solute transport by tubular epithelial cells. Tubular abnormalities observed in HNF-1β nephropathy include hyperuricemia with or without gout, hypokalemia, hypomagnesemia, and polyuria. Recent studies have identified novel p ost-transcriptional and post-translational regulatory mechanisms that control HNF-1β expression and activity, including the miRNA cluster miR17 ∼ 92 and the interacting proteins PCBD1 and zyxin. Further understanding of the molecular mechanisms upstream and downstream of HNF-1β may lead to the development of new therapeutic approaches in cystic kidney dise...
Source: Pediatric Nephrology - Category: Urology & Nephrology Source Type: research

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Publication date: Available online 14 July 2019Source: Pharmacological ResearchAuthor(s): Federica Raucci, Asif J. Iqbal, Anella Saviano, Paola Minosi, Marialuisa Piccolo, Carlo Irace, Francesco Caso, Raffaele Scarpa, Stefano Pieretti, Nicola Mascolo, Francesco MaioneAbstractGout is a paradigm of acute, self-limiting inflammation caused by the deposition of monosodium urate (MSU) crystals within intra-and/or peri-articular areas, which leads to excruciating pain, joint swelling and stiffness. The infiltration of leukocytes drives the inflammatory response and remains an attractive target for therapeutic intervention. In th...
Source: Pharmacological Research - Category: Drugs & Pharmacology Source Type: research
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MONDAY, July 15, 2019 -- More than one-fourth of patients with acute gout discharged from the emergency department receive an opioid prescription, according to a study published online July 2 in Arthritis Care&Research. Deepan S. Dalal, M.D.,...
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Publication date: October 2019Source: Phytochemistry, Volume 166Author(s): Bao-Bao Shi, Jing Chen, Mei-Fen Bao, Ying Zeng, Xiang-Hai CaiAbstractContinued interest in bioactive alkaloids led to the isolation of four undescribed alkaloids along with 74 known ones from the aerial parts of Tabernaemontana bufalina Lour. The structures of the yet undescribed alkaloids were elucidated based on NMR, IR, UV, MS and CD spectroscopic data and X-ray crystal diffraction and, according to the plant source, named as taberhaines A–D (1–4). The known compounds comprised of 66 monoterpenoid indole, three carboline and five isoq...
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Footwear is an important concern for people with gout, who often describe difficulty finding suitable footwear. Previous studies have identified footwear as a major concern for people with gout. The aim of thi...
Source: Journal of Foot and Ankle Research - Category: Podiatry Authors: Tags: Research Source Type: research
Nearly three in 10 patients seeking care at the emergency department (ED) for acute gout will be discharged with a prescription for opioids, new research shows.Reuters Health Information
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PMID: 31297604 [PubMed - as supplied by publisher]
Source: Zeitschrift fur Rheumatologie - Category: Rheumatology Authors: Tags: Z Rheumatol Source Type: research
ConclusionsIn the presence of elevated urate, human cartilage homogenates increase MSU crystal formation and promote the formation of smaller crystals which have greater inflammatory potential. These processes may contribute to the predilection of gout to osteoarthritic joints.This article is protected by copyright. All rights reserved.
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: Full Length Source Type: research
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